Fonollosa Alex, Carreno Ester, Adán Civera Alfredo, Dick Andrew D, Pellegrini Marco, Romano Francesco
Department of Ophthalmology, Biocruces Bizkaia Health Research Institute, Cruces University Hospital, University of the Basque Country, Barakaldo, Spain.
Department of Retina, Instituto Oftalmológico Bilbao, Bilbao, Spain.
Ocul Immunol Inflamm. 2025 Sep;33(7):1444-1450. doi: 10.1080/09273948.2024.2440563. Epub 2024 Dec 11.
To describe a case of early-stage Extensive Macular Atrophy with Pseudodrusen-like appearance (EMAP) presenting with acute monocular loss and atypical retinal lesions suggestive of posterior uveitis.
Case report with longitudinal follow-up including visual field testing and multimodal imaging.
A 53-year-old woman presented with sudden vision loss in her right eye (RE). Best corrected visual acuity (BCVA) was 20/400 in RE and unaffected in left eye (LE). Fundoscopy revealed bilateral pseudodrusen and peripheral paving-stone degeneration, consistent with stage 1 EMAP. Autofluorescence showed a hyper-autofluorescent reticular pattern in RE, colocalizing with pseudodrusen. Optical coherence tomography (OCT) demonstrated retinal pigment epithelium (RPE) thickening, RPE-Bruch's membrane separation, and atypical feathery lesions in the RE, along with ellipsoid (EZ) loss. In the LE, only pseudodrusen and RPE-Bruch's membrane separation were observed. Oral prednisone (30 mg, tapered) was initiated. At 24 weeks, the EZ had recovered, the feathery lesions resolved, and the RPE appeared normal, with a reduced hyper-autofluorescent pattern in the RE. At 30 weeks, the patient returned with recurrent vision loss in her RE. OCT revealed EZ loss without further lesions. After treatment with intravenous methylprednisolone and oral prednisone (tapered) plus methotrexate (15 mg/week), BCVA improved to 20/32, and EZ fully recovered.
We describe a case of stage 1 EMAP presenting with symptoms and lesions suggestive of concomitant Multiple Evanescent White Dot Syndrome (MEWDS)-like reaction. After treatment, resolution of inflammatory features and a partial improvement of the RPE abnormalities were observed.
描述一例早期广泛黄斑萎缩伴类玻璃膜疣样外观（EMAP），表现为急性单眼视力丧失及提示后葡萄膜炎的非典型视网膜病变。
病例报告及纵向随访，包括视野检查和多模态成像。
一名53岁女性右眼突发视力丧失。右眼最佳矫正视力（BCVA）为20/400，左眼未受影响。眼底检查发现双侧类玻璃膜疣及周边铺路石样变性，符合1期EMAP。自发荧光显示右眼有高自发荧光网状图案，与类玻璃膜疣共定位。光学相干断层扫描（OCT）显示右眼视网膜色素上皮（RPE）增厚、RPE- Bruch膜分离及非典型羽毛状病变，同时伴有椭圆体带（EZ）缺失。左眼仅观察到类玻璃膜疣和RPE- Bruch膜分离。开始口服泼尼松（30mg，逐渐减量）。24周时，EZ恢复，羽毛状病变消退，RPE外观正常，右眼高自发荧光图案减少。30周时，患者右眼视力再次丧失。OCT显示EZ缺失，无进一步病变。经静脉注射甲泼尼龙、口服泼尼松（逐渐减量）加甲氨蝶呤（15mg/周）治疗后，BCVA提高到20/32，EZ完全恢复。
我们描述了一例1期EMAP病例，其症状和病变提示伴有类似多发性一过性白点综合征（MEWDS）的反应。治疗后，炎症特征消退，RPE异常部分改善。
